Pedro Acien

Gynecology, Miguel Hernandez University of Elche
  • Miguel Hernandez University of Elche
  • Spain

Recent Comments

Jul 29, 2022

I have read with interest the article “Conservative surgical repair in cervical atresia associated with partial or complete absence of the vagina” by Fedele et al [1] and have also watched the attached video. I believe that both things (article and video) are probably inappropriate, and, in my opinion, it would be too risky conclude that “Laparoscopic-assisted uterovaginal/vestibular anastomosis may be considered the treatment of choice”. I would highlight the following points:

  1. There is no mention of associated anomalies or the need for their investigation in their three patients who are 14, 12 and 13 years old, especially when the latter two cases also had a unicornuate uterus together with cervico-vaginal atresia. Did they also have renal agenesis and dysplasia?
  2. It seems to me that the techniques described in text and showed in the video are inadequate, both in laparoscopy (uterine perforation, fundus hysterotomy) and vaginally (H Incision, “the edges of the uterine cavity were then anastomozed with the edges of the hymenal incision”).
  3. If there was cervical atresia, where was the “natural mucus” produced? What do you mean by "no stenosis of the neocervix was recorded"?
  4. As they did not use molds or prostheses and given the age of the patients (12-14 years), how did they maintain the neovagina functional? Did they have regular sexual activity?.

These comments are based on our clinical experience and on the cases and articles that I am citing in references [2-6].

Pedro Acién, MD, PhD, Emeritus Professor. Miguel Hernandez University, Campus of San Juan, Alicante, Spain. Email: acien@umh.es

References:

  1. Fedele L et al. Conservative surgical repair in cervical atresia associated with partial or complete absence of the vagina. Fertil Steril 2022, article in press
  2. Acién P et al. Cervicovaginal agenesis: spontaneous gestation at term after previous reimplantation of the uterine corpus in a neovagina. Hum Reprod 2008;23:548-553.
  3. Acién P et al. Unilateral cervico-vaginal atresia with ipsilateral renal agenesis. Eur J Obtet Gynecol Reprod Biol 2004;117:249-251.
  4. Acién P, Acién M. Malformations of the female genital tract and embryological bases. Current Women´s Health Reviews 2007;3:248-288.
  5. Acién P, Acién M. The presentation and management of complex female genital malformations. Hum Reprod Update 2016;22/1:48-69.
  6. Acién P et al. Clinical pilot study to evaluate the neovaginal PACIENA prosthesis® for vaginoplasty without skin grafts in women with vaginal agenesis. BMC Women's Health 2019;19:144. https://doi.org/10.1186/s12905-019-0841-z.
Nov 30, 2021

Comment for F&S-Dialog: https:// www.fertstertdialog.com/posts/33933

Pfeifer SM, Attaran M, Goldstein J, et al. ASRM müllerian anomalies classification 2021. Fertil Steril 2021;116/5:1238-1252.

Dear authors, we are surprised to read the new ASRM classification-2021 (1) and find out that it does not consider the concepts that we currently know on the genitourinary embryology and focuses only on morphological abnormalities, or imaging, of the Müllerian ducts, forgetting that in the embryonary development of the male and female genitourinary tract participates the entire urogenital ridge, the urogenital sinus and the Gubernaculum (2-4). We therefore presented an embryological and clinical classification system for malformations of the female genital tract (2-6) including 6 groups: 1) agenesis or hypoplasia of an entire urogenital ridge, 2) mesonephric anomalies, 3) müllerian anomalies, 4) Gubernaculum dysfunctions, 5) anomalies of the urogenital sinus, and 6) malformative combinations. In group 3 (isolated Müllerian anomalies) we included the AFS classification separating between anomalies of the paramesonephric or Müllerian ducts, the Müllerian anomalies tubercle, and the anomalies of both Müllerian tubercle and ducts (6).

The ASRM classification presented here might be useful for specialist in Diagnostic Imaging to dictate their Ultrasound, CT or MRI reports, but it does not seem suitable for the general clinician (including pediatricians, gynecologists and urologists) or for experts in Reproductive Medicine. In our opinion the image should induce an etiological and pathogenic diagnosis of the observed anomaly and also suggest possible associated anomalies.

The importance of embryology is such because unilateral renal agenesis (URA) is systematically associated with an ipsilateral genital abnormality in men and in women. Indeed, a distal anomaly of the mesonephric or Wolffian duct results in a triad of renal agenesis or hypoplasia (with eventual ectopic ureter in the blind vagina), uterine malformation (didelphys uterus, bicornuate, or less frequently, septate uterus) and ipsilateral blind or atretic hemivagina in women; whereas in men, it manifests by renal agenesis, seminal vesicle cyst and ejaculatory duct obstruction (Zinner syndrome), with eventual infertility (7).

And thus, considering the female genito-urinary embryology, we would say that:

The 5 images presented of Unicornuate Uterus can be associated with (right) renal agenesis: the first one clearly corresponds to patients with agenesis of all derivatives of the urogenital ridge on one side and there will usually also be extragenital (vertebral) anomalies; in 2, 3 and 4 there might be (right) renal agenesis if the uterine remnant, cavitated or not, is separated from the unicornuate uterus, and there could also be cases of mesonephric anomaly with unilateral (ipsilateral) cervico-vaginal agenesis; and number 5 is a communicating bicornuate uterus that also suggests right cervico-vaginal atresia. The same occurs in the Bicornuate Uterus number 2.

Of course, all anomalies with obstructed R/L hemivagina are mesonephric anomalies and have ipsilateral renal agenesis (or hypoplasia with eventual ectopic ureter), but there may also be partial resorption of the intervaginal septum and in that case the septum may be present only in the vaginal fundus and does not reach the lower third of the vagina.

In Complex Anomalies: number 1 would be a case of a Müllerian anomaly presenting a bicornuate uterus with bilateral cervical atresia and a normal simple vagina, which we believe is an unlikely situation being its possible etiology perhaps a segmental atresia. Number 2 is a case of communicating didelphys uterus with left cervico-vaginal atresia and as such

mesonephric anomaly, with renal agenesis (left). The figure number 3 we are not sure that the scheme is well presented but it may correspond to a mesonephric anomaly with a Gartner duct pseudocyst in the upper anterolateral wall of the vagina (as in Herlyn – Werner syndrome); number 4, according to the drawing, is an association of mesonephric anomaly with communicating bicornuate uterus (and cervico-vaginal atresia with right renal agenesis) and transverse vaginal septum or segmentary atresia (Müllerian tubercle anomaly). Number 5 is an isolated Müllerian (cervical) segmentary atresia.

So this pretended to be müllerian anomalies classification includes wolffian anomalies without acknowledging and does not mention or consider other malformations of the female genital tract such as urogenital sinus anomalies (congenital vesico-vaginal fistula and other cloacal anomalies), or the dysfunctions of the female Gubernaculum (as cavitated accessory uterine masses).

And in our opinion knowledge of the correct genitourinary embryology is essential for the understanding, study, diagnosis and subsequent treatment of genital malformations, especially complex ones and those that lead to gynecological and reproductive problems, particularly in young patients (8).

Pedro Acién, M.D., Ph.D., Emeritus Professor UMH. Department of Gynecology, Miguel Hernandez University of Elche, San Juan Campus, Alicante, Spain.

Maribel Acién, MD, PhD. Chief of Gynecology Section, San Juan University Hospital. Associate Professor. Department of Gynecology, Miguel Hernandez University of Elche, San Juan Campus, Alicante, Spain.

References:

1. Pfeifer SM, Attaran M, Goldstein J, et al. ASRM müllerian anomalies classification 2021. Fertil Steril 2021;116/5:1238-1252.

2. Acién P. Embryological observations on the female genital tract. Hum Reprod 1992;7/4:437-445.

3. Acién P, Acién M. Malformations of the female genital tract and embryological bases. Current Women´s Health Review 2007;3:248-88.

4. Acién P, Sánchez del Campo F. Mayol MJ, Acién M. The female gubernaculum: role in the embryology and development of the genital tract and in the possible genesis of malformations. Eur J Obstet Gynecol Reprod Biol 2011;159:426-432.

5. Acién P, Acién M, Sánchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod 2004;19/10:2377-84.

6. Acién P, Acién MI. The history of female genital tract malformation classifications and proposal of an updated system. Hum Reprod Update 2011;17/5:693-705.

7. Acién P, Acién M. Renal agenesis, associated genital malformations, and responsible genes (Reflections). Fertil Steril 2021;116/5:1370-71.

8. Acién P, Acién M. The presentation and management of complex female genital malformations. Hum Reprod Update 2016;22/1:48-69.

Nov 30, 2021

Comment for F&S-Dialog: https://www.fertstertdialog.com/posts/31809

Medvediev M et al. A case of neovagina surgical creation using the uterine cervix remnant in a patient with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 2021;116/5:1420-22.

Dear authors, in our opinion the reported case is not a Rokitansky or MRKH syndrome. In Rokitansky syndrome there is a complete utero-vaginal agenesis although bilateral Müllerian remnants that may eventually contain a functional endometrium might be present. Such anomaly corresponds to group I of the ASRM classification-2021 (1). Whereas, it seems clear that this patient had a congenital cervico-vaginal atresia or agenesis with a present and functional uterine body and normal tubes. The cervical agenesis seems to have been distal and the patient had previously undergone a subtotal (supracervical) hysterectomy due to pain and hematometra, leaving the cervical canal in which a mucinous cyst was later formed and that thereafter served to cover the neovagina (2). So the reported case, should be classified in group II.

Additionally, we support that it is not necessary to perform hysterectomy in cases of cervico-vaginal agenesis with a normal functional uterine body. Utero-neovaginal anastomosis can be done as can be seen in (3) and some other reports, with "good" results and retaining patient´s capability for conception.

Finally, neovagina can also be done following the McIndoe technique and the use of a PLA prosthesis without the need of skin graft (4,5).

Pedro Acién, M.D., Ph.D., Emeritus Professor. Department of Gynecology, Miguel Hernandez University of Elche, San Juan Campus, Alicante, Spain.

Maribel Acién, MD, PhD. Chief of Gynecology Section, San Juan University Hospital. Associate Professor. Department of Gynecology, Miguel Hernandez University of Elche, San Juan Campus, Alicante, Spain.

References:

1. Pfeifer SM, Attaran M, Goldstein J, et al. ASRM müllerian anomalies classification 2021. Fertil Steril 2021;116/5:1238-1252.

2. Medvediev M, Spesyvtsev D, Pokrovenko D. A case of neovagina surgical creation using the uterine cervix remnant in a patient with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 2021;116/5:1420-22.

3. Acién P, Acién MI, Quereda F, Santoyo T. Cervicovaginal agenesis: spontaneous gestation at term after previous reimplantation of the uterine corpus in a neovagina: Case Report. Hum Reprod 2008;23/3:548-553.

4. Acién P, Acién M, Oliva-Meyer MA, Sánchez-Lozano M, Martínez-Garcia J. McIndoe Operation without Skin Graft: A New Neovaginal Prosthesis of Polylactic Acid (PLA). British Journal of Medicine & Medical Research 2016;18(9):1-7.

5. Acién P, Nohales F, Sánchez-Ferrer ML, Sánchez-Lozano M, Navarro-Lillo V, Acién MI. Clinical Pilot Study to Evaluate the Neovaginal PACIENA Prosthesis® for Vaginoplasty without Skin Graft in Women with Vaginal Agenesis. BMC Women´s Health, 2019, Nov 26;19(1):144.